Pathology Department, Faculty of Medicine, Tanta University, Tanta, Egypt.
J Egypt Natl Canc Inst. 2021 Feb 8;33(1):6. doi: 10.1186/s43046-021-00060-w.
Medulloblastoma (MB) is a heterogeneous disease, displaying distinct genetic profiles with specific molecular subgroups. This study aimed to validate MB molecular subgrouping using surrogate immunohistochemistry and associate molecular subgroups, histopathological types, and available clinicopathological parameters with overall survival (OS) and progression-free survival (PFS) of MB patients. This study included 40 MBs; immunohistochemical staining, using β-catenin and GRB2-Associated Binding Protein 1 (GAB1) antibodies, was used to classify MB cases into wingless signaling activated (WNT), sonic hedgehog (SHH), and non-WNT/SHH molecular subgroups. Nuclear morphometric analysis (for assessment of degree of anaplasia) and Kaplan-Meier survival curves were done.
MB cases were classified into WNT (10%), SHH (30%), and non-WNT/SHH (60%) subgroups. Histopathological types differed significantly according to tumor location (p< 0.001), degree of anaplasia (p = 0.014), molecular subgroups (p < 0.001), and risk stratification (p = 0.008). Molecular subgroups differed significantly in age distribution (p = 0.031), tumor location (p< 0.001), histopathological variants (p < 0.001), and risk stratification (p < 0.001). OS was 77.5% and 50% after 1 and 2 years, while PFS was 65% and 27.5% after 1 and 2 years, respectively. OS and PFS were associated significantly with histopathological variants (p < 0.001 and 0.001), molecular subgroups (p = 0.012 and 0.005), and risk stratification (p < 0.001 and < 0.001), respectively.
Medulloblastoma classification based on molecular subgroups, together with clinicopathological indicators, mainly histopathological types; accurately risk stratifies MB patients and predicts their survival.
髓母细胞瘤（MB）是一种异质性疾病，具有不同的遗传特征，存在特定的分子亚群。本研究旨在通过替代免疫组织化学方法验证 MB 分子亚群分类，并将分子亚群、组织病理学类型以及现有的临床病理参数与 MB 患者的总生存（OS）和无进展生存（PFS）相关联。本研究纳入了 40 例 MB 患者；使用 β-连环蛋白和 GRB2 相关结合蛋白 1（GAB1）抗体进行免疫组织化学染色，将 MB 病例分为无翅信号激活（WNT）、声波刺猬（SHH）和非 WNT/SHH 分子亚群。进行核形态计量分析（用于评估间变程度）和 Kaplan-Meier 生存曲线。
MB 病例分为 WNT（10%）、SHH（30%）和非 WNT/SHH（60%）亚群。组织病理学类型根据肿瘤位置（p<0.001）、间变程度（p=0.014）、分子亚群（p<0.001）和风险分层（p=0.008）差异显著。分子亚群在年龄分布（p=0.031）、肿瘤位置（p<0.001）、组织病理学变异型（p<0.001）和风险分层（p<0.001）方面差异显著。OS 分别为 1 和 2 年后的 77.5%和 50%，而 PFS 分别为 1 和 2 年后的 65%和 27.5%。OS 和 PFS 与组织病理学变异型（p<0.001 和 0.001）、分子亚群（p=0.012 和 0.005）和风险分层（p<0.001 和 <0.001）显著相关。
基于分子亚群的髓母细胞瘤分类，结合临床病理指标，主要是组织病理学类型，可准确地对 MB 患者进行风险分层，并预测其生存。
